A countrywide search for Creutzfeldt-Jakob disease in Israel disclosed 29 cases with onset between 1963 and 1972. Incidence in various ethnic groups varied in the narrow range of 0.4 to 1.9 per million population except among Jewish immigrants from Libya, among whom the incidence was 31.3 per million. An extraordinary excess of Creutzfeldt-Jakob disease exists in this ethnic group.

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A case of progressive hemifacial atrophy (PHA) with extensive atrophy of the walls of the maxillary sinus is reported. Prolapse of subcutaneous tissue and orbital fat through the bony defect simulated the presence of a tumour and led to surgical exploration. Such a severe degree of bony involvement with relatively mild soft tissue atrophy is exceptional. Radiological evaluation by hypocycloidal tomography contributed greatly to the diagnosis in this case.Unilateral progressive atrophy of the face was first described by Parry (1825) and by Romberg (1846). Eulenberg (1871) coined the name ‘progressive facial hemiatrophy’, but ‘progressive hemifacial atrophy’ (PHA) is a more appropriate term (Gorlin and Pindborg, 1964; Kabir and Halprin, 1964). The disease commences usually in the first or second decade of life, and is more common in females. Over several years there is a slowly progressive atrophy of the face on one side, with involvement of the skin and subcutaneous fat, and on rare occasions also of the muscles and bones (Gorlin and Pindborg, 1964; Finesilver and Rosow, 1938; Bramley and Forbes, 1960; Singh and Bajpai, 1969; Crikelair et al., 1962). The etiology of the disease is unknown.We report an unusual case in which the hemifacial atrophy affected the bone much more than the soft tissues and which presented as a mass in the maxillary sinus.

T h e foreign bodies usi, ally encountered in the brain are bullets, splinters of metal or fragments of bone resulting from direct blows on the head. As far as we know, however, there has been no previous report of a sewing needle having been found in t h e brain.We have recently come across ~ examples of this heurosurgical curiosity; t h e needles were t h o u g h t to have been in place ~3 a n d 54 years respectively. Permission for surgery was obtained in 1 case a n d t h e needle was extracted successfully. CASE REPORTSCase 1. A ~3-year-old woman began to suffer from fits 5 years previously, when she suddenly lost consciousness, with convulsions and incontinence of urine. Six months later she had a second attack and subsequently the fits recurred with increasing frequency up to once or twice a month.On the day of the attack she usually suffered from right parieto-oecipital headaches. Therapy with barbiturates was ineffective and she was referred to hospital because of worsening of her condition with four attacks in the previous ~ weeks.The general clinical and neurological findings, including eyegrounds, were quite normal, as were the usual laboratory tests and the cerebrospinal fluid.Roentgenograms of the skull showed a sewing needle in the right parietal lobe of the brain (Fig. 1); one extremity lay close to the inner table of the skull and in its vicinity a small bony defect could be seen clearly indi-caring the route of entry (Fig. ~). Electroencephalography revealed a very few low-voltage sharp waves in the right parietal region, not certainly of pathological significance against a dysrhythmic background devoid of generalized paroxysmal features.Right parietal craniectomy was performed under general anesthesia at the site of the puncture marking the point of entry of the needle. When the bone was exposed, this was seen as a hole 1-~ mm. in diameter covered by connective tissue which was bound to the dura mater. On incising the dura mater at this spot, the head of the needle came into view, projecting for about 1 mm. from the cortex of the brain which was stained with dark pigment in the neighborhood over a diameter of about 1ı89 cm. The needle itself lay in a narrow cystic cavity a few mm. wide. It was removed with ease ( Fig. 3) and the surrounding cyst was emptied. The needle was covered by a layer of rust. It was found on chemical examination to be made of steel.The patient was instructed to continue taking barbiturates after the operation; during the 5 months that have passed, there have been no further fits. A recent electroencephalogram was normal.Case 2. A 54-year-old woman was referred because of left-sided headache, from which she had suffered for the previous ~ years; in recent months she had complained also of a disturbance of vision.Neurological findings were normal except for an incomplete right inferior homonymous quadrantanopia. The electroencephalogram was normal. Roentgenograms of the skull showed a sewing needle in the left FIo. 1. Case 1. Roentgenogram showing needle situated in right ...

(Mulhausen et al., 1967) and may stimulate lactate production by erythrocytes and muscle. In the presence of hypoxia hepatic anaerobic glycolysis may occur, resulting in lactate production rather than removal (Berry and Scheuer, 1967). Leppla et al. (1964) (Heinz et al., 1968), and, secondly, the subsequent steps in fructose utilization are largely independent of phosphofructokinase, whereas this enzyme regulates the rate of glucose metabolism after initial phosphorylation. As a result of rapid fructose breakdown the end products of glycolysis, lactate and pyruvate, accumulate in the blood (Bergstrom et al., 1968) and the liver may be depleted of enough ATP and inorganic phosphate to reduce protein synthesis to an important extent, thereby impairing the integrity of enzyme systems concerned with lactate removal (Maenpaa et al., 1968).Magnesium deficiency has been reported in chronic alcoholism and cirrhosis (Flink, 1956) and in hepatic coma. Since many factors concerned with carbohydrate metabolism-in particular coenzyme A (Flink, 1956)-are magnesium dependent magnesium depletion may impair utilization of lactate, but the accumulation of lactate in the present case suggests that it does not impede lactate formation.We believe that in this patient lactate formation resulting from fructose breakdown initiated the lactic acidosis, since this coincided with fructose therapy and improved slightly when glucose was substituted and the serum magnesium was corrected. The data of Mulhausen et al. (1967) show that once established in liver failure lactic acidosis is often irreversible. We feel that fructose is contraindicated in the treatment of liver failure and suggest that ethanol-fructose mixtures should be used with caution if hepatic function is disturbed, since ethanol can also induce lactic acidosis by altering the proportion of NADH to NAD in the liver cell.We wish to thank Dr. J. Whitfield, of the renal unit, Queen Elizabeth Hospital, for kindly carrying out the lactate analyses.